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Inherited and environmental

Long QT syndrome

A QTc beyond 480 ms — the substrate for torsades, whether inherited or drug-induced.

60 bpmSinus, QTc 520 ms
II
II. The T wave is broad and late; the QT occupies more than half the RR interval.

What you see

  • QTc over 450 ms in men or 460 ms in women; over 500 ms is high risk.
  • Broad, late or notched T waves.
  • T wave alternans, when present, signals imminent arrhythmia.
  • A useful bedside check at normal rates: the QT should be less than half the RR interval.

Why it looks like that

Repolarising potassium current is reduced — by a channel mutation in congenital forms, or by drug blockade of the hERG channel in acquired ones. Repolarisation is prolonged and non-uniform, allowing early afterdepolarisations to trigger torsades.

What to do

  • Stop every QT-prolonging drug; correct potassium, magnesium and calcium.
  • Beta-blockade (propranolol or nadolol) for congenital long QT; avoid competitive sport in LQT1.
  • ICD for those who have had cardiac arrest or syncope despite beta blockade.
  • Screen and counsel first-degree relatives.

The trap

Use Bazett's correction with care: it over-corrects at fast rates and under-corrects at slow ones, so a tachycardic patient can be labelled with a long QT they do not have. Also remember the trigger pattern — LQT1 with exercise and swimming, LQT2 with startle and emotion, LQT3 during sleep.

QTcBazetthERGbeta-blocker

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